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Abstract
Streptococcal toxic shock syndrome (TSS) is a fulminant disease characterised by rapid progression to multiorgan failure. However, streptococcal TSS manifesting as uterine necrosis (UN) is unusual. Here, we present a case of a woman in her 30s with a constellation of symptoms, including abdominal pain, fatigue and fever. Despite an initially unclear clinical picture, she rapidly developed shock with radiographical evidence of intrabdominal arterial narrowing with spurious multiorgan infarctions and ischaemic colitis. Exploratory laparotomy uncovered unexpected UN requiring hysterectomy. Multiple complications ensued, marked by shock liver, acute renal failure, cutaneous desquamation and distal gangrene. This case highlights a unique presentation of streptococcal TSS as UN and emphasises the rapidly deteriorating nature of the disease.
- Adult intensive care
- Infectious diseases
- Obstetrics and gynaecology
- Purpura Fulminans
- Uterus
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Background
Streptococcal toxic shock syndrome (TSS) is an acute, often-fatal complication of invasive group A streptococcal (GAS) disease mediated by the production of super-antigenic toxins.1 The bacterium most commonly enters through the skin, vagina and pharynx. However, the absence of a discernible entry point does not preclude a diagnosis of streptococcal TSS, as identifiable portal of entry is often unfound.2 The clinical presentation of streptococcal TSS is highly variable but often begins with flu-like symptoms such as fever, myalgia and nausea. Hypotension typically develops within the first 24 to 48 hours, followed by the characteristic progression to shock with multiorgan failure.
In a similar nature, uterine necrosis (UN) is a rare, life-threatening condition; few cases have been described as complications of uterine artery embolisation for postpartum haemorrhage, caesarean section or severe endometritis.3 Although streptococcal TSS is an established cause of necrotising infections, to our knowledge, UN is a novel presentation not previously identified in literature. Here, we describe the case of a patient presenting with shock who was found to have UN and streptococcal TSS despite the absence of known risk factors. While rare, the report of serious GAS infections is essential to serve as reminder of the devastating and fatal sequelae of the disease.
Case presentation
A woman in her 30s with a history of psoriasis presented to the emergency department with abdominal pain, fatigue and fever. 1 week prior to the presentation, she developed lower abdominal cramping with profound fatigue and anorexia. She had three episodes of diarrhoea and took ibuprofen for flu-like symptoms for the past day. On the morning of admission, her abdominal pain became unrelenting, and she developed a fever of 101 °F prompting her visit.
She has a medical history of psoriasis treated with topical therapy only. She was sexually active with her husband who had undergone prior vasectomy. Her last menstrual period was over 2 weeks ago, and she was not using tampons or pads.
Initial vitals showed blood pressure of 70/38 mm Hg, heart rate of 111 beats per minute, respiratory rate of 23 breaths per minute and temperature of 97.5 °F. Physical examination was notable for dry mucous membranes, normal cardiac sounds without murmurs, clear lungs and diffuse abdominal tenderness with guarding without rebound. Her lower extremities were cool and mottled with delayed capillary refill, but the skin was intact without erythema, rash or blistering. Her Glasgow Coma Score was 15, and no nuchal rigidity or focal neurological deficits were present. The initial laboratory results obtained in the emergency department are delineated in table 1.
Laboratory results obtained in the emergency department
Investigations
Her blood pressure normalised following adequate fluid resuscitation, and broad-spectrum antibiotics were initiated after blood cultures were obtained. A CT angiography (CTA) in the chest, abdomen and pelvis showed severe arterial narrowing of coeliac, superior mesenteric, bilateral renal and left external iliac arteries along with extensive oedema and pericolonic fat-stranding of the sigmoid colon and rectum (figure 1). Infarction of bilateral kidneys and bilateral adrenal glands was also noted (figure 2). A transthoracic echocardiogram revealed a left ventricular ejection fraction of 35% without valvular abnormalities. Due to concern for ischaemic colitis, she underwent emergent exploratory laparotomy which showed normal intra-abdominal organs without evidence of infarction or ischaemic bowel. However, further inspection of the pelvis unexpectedly revealed frank necrosis in the uterus, fallopian tubes and ovaries. Consequently, a hysterectomy with bilateral salpingo-oophorectomy was performed, and uterine tissue was sent for histopathology and culture (figure 3).
CTA of the abdomen and pelvis showing (A) extensive oedema and pericolonic fat-stranding of the sigmoid colon and rectum and (B) severe arterial narrowing of the left external iliac artery. CTA, CT angiography.
CTA of the abdomen and pelvis showing numerous wedge-shaped, hypo-enhancing regions throughout bilateral kidneys in the (A) coronal section and (B) transverse section. CTA, CT angiography.
Macroscopic view of gross hysterectomy specimen with bilateral adnexa from anatomical posterior view (A) and anterior view (B). The ovaries, fallopian tubes and uterus were found to be dusky intraoperatively consistent with devascularisation and necrosis requiring complete resection.
The following day, the patient’s clinical condition continued to deteriorate. Her hypotension worsened leading to increasing vasopressor support, and she was intubated for respiratory failure. Despite resuscitation efforts, she went into cardiac arrest with the development of shock liver and worsening renal failure requiring renal replacement therapy. At that point, blood and intraoperative uterine tissue cultures yielded Streptococcus pyogenes.
Differential diagnosis
The patient’s presentation of hypotension, thrombocytopenia, lactic acidosis, elevated procalcitonin and radiographical evidence of multiorgan infarction was suspicious for disseminated intravascular coagulation in the setting of septic shock. However, exploratory laparotomy revealed grossly normal colon, kidneys and adrenal glands. Consequently, the transient stenosis of various arteries was attributed to multifocal vasospasms due to septic shock.
The subsequent isolation S. pyogenes, hypotension, renal impairment, liver involvement and coagulopathy confirmed the diagnosis of streptococcal TSS.
Treatment
The patient was diagnosed with streptococcal TSS and administered intravenous immune globulin 1 g/kg once, followed by 500 mg/kg daily for 3 days. Additional investigations including PCR for chlamydia and gonorrhoea were negative. Efforts to narrow antibiotics to penicillin G 12 million units every 12 hours and clindamycin 800 mg every 8 hours to complete a 14-day course were hindered by recurrent shock and fever. Subsequently, she developed cutaneous desquamation of approximately 50–80% of total body surface area and distal dry gangrene of bilateral upper and lower extremities prompting transfer to a specialised burn unit for ongoing complex wound care (figure 4). Despite multiple rounds of surgical debridement and skin grafting, her extremities developed diffuse necrotic eschars which ultimately required bilateral above-knee amputations of the lower extremities and bilateral transradial amputations of the upper extremities.
(A,B) Acute purpuric rash of the right upper extremity, with irregular demarcated areas of necrosis. Areas of erythema and haemorrhage are also visualised. Transferred to burn unit for attempts at debridement, but necrosis extended to muscle and eventually required amputation along with other three extremities.
Outcome and follow-up
Her intensive care unit course was further complicated by heparin-induced thrombocytopenia requiring argatroban and Trichosporon fungemia treated with isavuconazole. Currently, the patient has made a full recovery. Due to the amputation of all four extremities, she requires her family members with a wheelchair to assist with her activities of daily living. However, she is scheduled for prosthesis fitting for both the upper and lower extremities, and she considers herself very fortunate to be alive.
Discussion
Streptococcal TSS is a complication of invasive GAS infection characterised by multiorgan failure. The estimated incidence of invasive GAS infection is 3.5 cases per 100 000 persons.4 Moreover, the case-fatality rate of approximately 30–60% has remained unchanged in recent decades despite medical advancements.4 Pre-existing skin lesions are the most frequently identified risk factor for invasive GAS infection. Alcohol abuse, chronic lung disease, immunosuppression, intravenous drug use, heart disease, diabetes, cancer, varicella zoster virus infection and recent childbirth have also been identified as risk factors. While streptococcal TSS predominantly affects individuals with predisposing risk factors, the recent increase in the frequency of disease among patients 20 to 35 years of age is thought to be due to pregnancy-associated infections and deep infections following muscle injury.4
The transmission of GAS occurs via entry through compromised barriers like skin or mucosal membranes. Psoriasis could have acted as an entry portal in this patient. Surgical wounds and vaginal mucosa are also common sites, but the route of entry remains unknown for 50% of cases.5 Following entry, GAS releases streptococcal toxins, triggering an inflammatory cascade with subsequent capillary leakage and tissue injury. Invasive GAS infections including bacteraemia, sepsis and necrotising fasciitis can be devastating with high mortality rates worldwide.6 Furthermore, invasive GAS infections carry a higher risk of TSS with an estimated risk of necrotising fasciitis causing TSS to be near 50%. However, the incidence of streptococcal TSS in association with UN is unique and, to our knowledge, has not been documented in medical literature.5 7
The rarity of this presentation may be attributed to the pathogenesis of the UN. Of the few documented cases of UN, most were complications of postpartum haemorrhages, septic abortion, severe endometritis or B-lynch suture with vascular ligation (Kaul). However, data on infectious aetiologies of UN is limited. In literature, streptococcal TSS has been associated with intrauterine infections in women who are in the second and third trimester of pregnancy.8 Moreover, cases of streptococcal TSS due to gynaecological surgery such as caesarean sections further illustrate the elevated risk during peripartum period.9 Untreated pelvic inflammatory disease can lead to UN, but testing for Neisseria gonorrhoeae and Chlamydia trachomatis was negative in our patient.9 In our patient, who had no evidence of foetal remnants on uterine tissue histopathology, history of gynaecological infection or recent surgery, it was initially suspected that her psoriasis provided a portal of entry for GAS. Psoriasis has no known association with invasive GAS infection of the uterus or adnexa; however, Corynebacterium striatum tubo-ovarian abscess leading to septic shock has been previously reported.10 In our patient, the exact method of GAS dissemination leading to her UN remains unclear.
The clinical presentation of streptococcal TSS ranges from soft-tissue infection to sudden-onset fever, rash, hypotension and systemic signs of infection. Preceding flu-like symptoms of fever, myalgia and emesis occur in 20% of patients.11 As seen in our patient, the subsequent development of myonecrosis and sloughing of skin can be seen within 24 to 72 hours from presentation. Dermatopathological findings are variable and should be used in context with other clinical findings to confirm the diagnosis of TSS. The clinical feature of streptococcal TSS that is most distinct from other causes of multiorgan failure is the rapid progression of shock, typically within hours from presentation.11 The severity of end-organ damage may initially seem out of proportion to or precede the onset of hypotension. Given the vague initial presentation of group A streptococcus, its rapid progression to toxic shock syndrome and potential multi-organ involvement necessitates maintaining a high index of suspicion and keeping this disease at the forefront of clinical consideration, making prompt recognition of organ failure critical to early diagnosis and treatment.
Learning points
Consider streptococcal toxic shock syndrome (TSS) as a differential diagnosis in patients presenting with rapidly progressive shock, irrespective of the presence of identifiable causes or risk factors.
Recognise the heterogeneous and non-specific clinical manifestations of TSS, understanding that patients may deteriorate rapidly despite prompt and intensive treatment.
Reveal the progressive nature of streptococcal TSS and its numerous potential complications, emphasising the need for a multidisciplinary approach to management.
Identify common aetiologies and risk factors associated with uterine necrosis and that urgent or emergent hysterectomy is paramount to definitive treatment.
Ethics statements
Patient consent for publication
Footnotes
Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms and critical revision for important intellectual content: MS, NB, VK and DP. The following authors gave final approval of the manuscript: MS, NB, VK and DP. DP acted as the guarantor.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.